What is ISNA?

ISNA, the Intersex Society of North America, is a peer support, education,
and activist group operated by and for intersexuals. We are pleased to
have the counsel of three professional sexologists who serve on our board
of directors.

ISNA is a not-for-profit, unincorporated organization, and our operations
are funded by donations. Send check or money order, payable to ISNA, to
PO Box 31791 San Francisco CA 94131.

What are the activities of ISNA?

ISNA publishes a quarterly newsletter called Hermaphrodites with Attitude.
Subscriptions are available for $12 ($18 overseas). We strive to make information
about intersexuality available to the general public and especially to
educators and medical and mental health professionals. We hold regularly
scheduled peer support group meetings in the San Francisco Bay Area. We
make introductions for intersexuals located outside the Bay Area, so that
they may correspond by post, email, or telephone.

What on-line info does ISNA make available?

ISNA's web site, which came into operation in January 1996, offers our
quarterly newsletter, Hermaphrodites with Attitude, informational pamphlets
about various intersex conditions, answers to frequently asked questions,
and bibliographies relating to intersexuality.

What print info does ISNA make available?

In addition to our quarterly newsletter, ISNA plans to publish informational
pamphlets about a variety of intersex conditions. Currently, the following
pamphlets are available by post. Please send $1 per pamphlet, $3 per bibliography
(payable to ISNA) for shipping and handling (for any combination or all
four).

Pamphlets ($1 each)

Recommendations for Treatment: Intersex infants and children

Hypospadias Surgery: A parents' guide

Bibliographies ($3 each)

Annotated Bibliography

Intersexuality: A Guide to Research (a 14 page bibliography, not annotated)

In addition, back issues of Hermaphrodites with Attitude are available
for $5 each. The following issues are available:

What is intersexuality (or hermaphroditism)?

Our culture conceives sex anatomy as a dichotomy: humans come in two sexes,
conceived of as so different as to be nearly different species. However,
developmental embryology, as well as the existence of intersexuals, proves
this to be a cultural construction. Anatomic sex differentiation occurs
on a male/female continuum, and there are several dimensions.

Genetic sex, or the organization of the "sex chromosomes," is commonly
thought to be isomorphic to some idea of "true sex." However, something
like 1/500 of the population have a karyotype other than XX or XY. Since
genetic testing was instituted for women in the Olympic Games, a number
of women have been disqualified as "not women," after winning. However,
none of the disqualified women is a man; all have atypical karyotypes,
and one gave birth to a healthy child after having been disqualified.

The sex chromosomes determine the differentiation of the gonads into
ovaries, testes, ovo-testes, or nonfunctioning streaks. The hormones produced
by the fetal gonads determine the differentiation of the external genitalia
into male, female, or intermediate (intersexual) morphology. Genitals develop
from a common precursor, and therefore intermediate morphology is common,
but the popular idea of "two sets" of genitals (male and female) is not
possible. Intersexual genitals may look nearly female, with a large clitoris,
or with some degree of posterior labial fusion. They may look nearly male,
with a small penis, or with hypospadias. They may be truly "right in the
middle," with a phallus that can be considered either a large clitoris
or a small penis, with a structure that might be a split, empty scrotum,
or outer labia, and with a small vagina that opens into the urethra rather
than into the perineum.

What is androgen insensitivity syndrome?

Androgen Insensitivity Syndrome, or AIS, is a genetic condition, inherited
(except for occasional spontaneous mutations), occurring in approximately
1 in 20,000 individuals. In an individual with complete AIS, the body's
cells are unable to respond to androgen, or "male" hormones. ("Male" hormones
is an unfortunate term, since these hormones are ordinarily present and
active in both males and females.) Some individuals have partial
androgen insensitivity.

In an individual with complete AIS and karyotype 46 XY, testes develop
during gestation. The fetal testes produce mullerian inhibiting hormone
(MIH) and testosterone. As in typical male fetuses, the MIH causes the
fetal mullerian ducts to regress, so the fetus lacks uterus, fallopian
tubes, and cervix plus upper part of vagina. However, because cells fail
to respond to testosterone, the genitals differentiate in the female, rather
than the male pattern, and Wolffian structures (epididymis, vas deferens,
and seminal vessicles) are absent.

The newborn AIS infant has genitals of normal female appearance, undescended
or partially descended testes, and usually a short vagina with no cervix.
Occasionally the vagina is nearly absent. AIS individuals are clearly women.
At puberty, the estrogen produced by the testes produces breast growth,
though it may be late. She does not menstruate, and is not fertile. Most
AIS women have no pubic or underarm hair, but some have sparse hair.

When an AIS girl is diagnosed during infancy, physicians often perform
surgery to remove her undescended testes. Although removal of testes is
advisable, because of the risk of cancer, ISNA advocates that surgery be
offered later, when the girl can choose for herself. Testicular cancer
is rare before puberty.

Vaginoplasty surgery is frequently performed on AIS infants or girls
to increase the size of the vagina, so that she can engage in penetrative
intercourse with a partner with an average size penis. Vaginoplasty surgery
is problematic, with many failures. ISNA advocates against vaginal surgery
on infants. Such surgery should be offered to, not imposed on, the pubertal
girl, and she should have an opportunity to speak with adult AIS women
about their sexual experience and about surgery in order to make a fully
informed decision. Not all AIS women will choose surgery.

Some women have successfully increased the depth of their vagina with
a program of regular pressure dilation, using aids designed for that purpose.
Contact the AIS Support Network (See What are related
support groups?) for more information.

Physicians and parents have been most reluctant to be honest with AIS
girls and women about their condition, and this secrecy and stigma has
unnecessarily increased the emotional burden of being different.

Because AIS is a genetic defect located on the X chromosome, it runs
in families. Except for spontaneous mutations, the mother of an AIS individual
is a carrier, and her XY children have a 1/2 chance of having AIS. Her
XX children have a 1/2 chance of carrying the AIS gene. Most AIS women
should be able to locate other AIS women among siblings or maternal relatives.

Is there a test for androgen insensitivity syndrome?

The answer depends upon exactly what you are looking for--diagnostic information,
or carrier status. If were born with female genitals and testes, and have
very sparse or absent pubic hair, you most likely have complete AIS. If
you were born with ambiguous genitals and testes, there are a number of
possible etiologies, including partial AIS.

Testing for partial AIS is more problematic than the complete form.
Hormonal tests in a newborn with 46 XY karyotype and ambiguous genitals
will show normal to elevated testosterone and LH, and a normal ratio of
testosterone to DHT. A family history of ambiguous genitals in maternal
relatives suggests partial androgen insensitivity.

If you are wondering if you are a carrier, or if you know that you are
a carrier and are wondering about the status of your fetus, genetic testing
is possible. AIS has been diagnosed as early as 9-12 weeks gestation by
chorionic villus sampling (sampling tissue from the fetal side of the placenta).
By the 16th week it can be detected by ultrasound and amniocentesis. However,
prenatal diagnosis is not indicated unless there is a family history of
AIS.

What is partial androgen insensitivity syndrome?

The extent of androgen insensitivity in 46 XY individuals is quite variable,
even in a single family. Partial androgen insensitivity typically results
in "ambiguous genitalia." The clitoris is large or, alternatively, the
penis is small and hypospadic (these are two ways of labeling the same
anatomical structure). Partial androgen insensitivity may be quite common,
and has been suggested as the cause of infertility in many men whose genitals
are of typically male appearance.

Individuals with ambiguous genitals have typically been subjected to
"corrective" surgery during infancy. Based on our own painful experiences,
ISNA believes that such cosmetic surgery of the genitals is harmful and
unethical. Surgery is justified only when it is necessary for the health
and well-being of the child. Surgery which is intended to make the genitals
appear more male or more female should be offered, but not imposed, only
when the child is old enough to make an informed decision for her/himself.

What is Progestin Induced Virilization?

Caused by prenatal exposure to exogenous androgens, most commonly progestin.
Progestin is a drug which was administered to prevent miscarriage in the
50's and 60's and it is converted to an androgen (virilizing hormone) by
the prenatal XX persons metabolism. If the timing is right, the genitals
are virilized with effects ranging from enlarged clitoris to the development
of a complete phallus and the fusing of the labia. In all cases ovaries
and uterus or uterine tract are present, though in extreme cases of virilization
there is no vagina or cervix, the uterine tract being connected to the
upper portion of the urethra internally. The virilization only occurs prenatally
and the endocrinological functionality is unchanged, ie. feminizing puberty
occurs due to normally functioning ovaries.

In other words, XX people affected in-utero by virilizing hormones can
be born into a continuum of sex phenotype which ranges from "female with
larger clitoris" to "male with no testes". It is noteworthy that the use
of progestin is not effective in the prevention of miscarriage.

Progestin androgenized children are subjected to the same surgically
enforced standards of cosmetic genital normalcy as other intersexed children...
meaning that clitoridectomy and possibly more extensive procedures are
often performed early in life, most often with the effect of loss of erotic
sensation and ensueing psychological trauma. ISNA believes that this surgery
is unneccessary, cosmetic and primarily "cultural" in its significance.
It is of no benefit to the child, who suffers even more from the stigma
and shame of having been surgically altered than she would have had her
non-standard genitals been allowed to remain intact.

Occasionally a female neonate will be so genitally virilized that she
is given a male identity at birth and raised as a boy. It is important
not to hide the circumstances of her biology from such a child, in order
to the avoid shame, stigma and confusion which results from secrecy. After
the onset of puberty the child may want to explore the option, hopefully
with the aid of loving parents and peer counseling, of having surgery to
allow expression of either female or male sexuality. This is not a choice
that should be forced prematurely, it is a personal choice to be made by
a teenager about his/her body and about her/his choice of sexual identity
and sexuality.

What is Adrenal Hyperplasia?

Adrenal Hyperplasia is the most prevalent cause of intersexuality amongst
XX people with a frequency of about 1 in 20000 births. It is caused when
an anomoly of adrenal function (usually 21-hydroxylase or 11-hydroxylase
deficiency) causes the synthesis and excretion an androgen precursor, initiating
virilization of a XX person in-utero. Because the virilization originates
metabolically, masculinizing effects continue after birth.

As in progestin induced virilization, sex phenotype varies along the
same continuum, with the possible added complication of metabolic problems
which upset serum sodium balance. The metabolic effects of CAH can be counteracted
with cortisone. The scenario for medical intervention for intersex is similar...
but CAH people have an increased likelihood of early detection due to metabolic
imbalances (Salt Losing Form). The long term use of cortisone itself produces
significant dependance and other side effects, all of which need to be
explained honestly and openly.

What is klinefelter syndrome?

Most men inherit a single X chromosome from their mother, and a single
Y chromosome from their father. Men with klinefelter syndrome inherit an
extra X chromosomes from either father or mother; their karyotype is 47
XXY. Klinefelter is quite common, occuring in 1/500 to 1/1,000 male births.

The effects of klinefelter are quite variable, and many men with klinefelter
are never diagnosed. The only characteristic that seems certain to be present
is small, very firm testes, and an absence of sperm in the ejaculate, causing
infertility. Except for small testes, men with klinefelter are born with
normal male genitals. But their testes often produce lower than average
quantities of testosterone, so they don't virilize (develop facial and
body hair, muscles, deep voice, larger penis and testes) as strongly as
other boys at puberty. Many also experience some gynecomastia (breast growth)
at puberty.

Physicians recommend that boys with klinefelter be given testosterone
at puberty, so that they will virilize in the same way as their peers,
and that men with klinefelter continue to take testosterone thoughout their
lives, in order to maintain a more masculine appearance and high libido.
Many ISNA members, however, report that they do not like the effects of
testosterone, and prefer to reduce their dosage, or not to take it at all.

Many ISNA members with klinefelter syndrome are homosexual, a few are
transsexual, and nearly all experience their gender as quite different
from other men. In contrast, medical literature tends to discount any connection
between klinefelter syndrome and homosexuality or gender issues. We suspect
that medical reassurances that "your son will not be gay" are based more
on homophobia than on an accurate assessment of probabilities. Gay children
deserve honesty and parental love and support!

What is hypospadias?

Hypospadias refers to a urethral meatus ("pee-hole") which is located along
the underside, rather than at the tip of the penis. In minor, or distal
hypospadias, the meatus may be located on the underside of the penis, in
the glans. In more pronounced hypospadias, the urethra may be open from
mid-shaft out to the glans, or the urethra may even be entirely absent,
with the urine exiting the bladder behind the penis.

What are the frequencies
of intersex conditions?

The following statistics are approximate, and are stated in terms of the
frequency per 1000 live births. Note that the frequency of inherited genetic
conditions, such as congenital adrenal hyperplasia, differs for different
populations. The statistics presented here are our best guess for North
America. Our appreciation to Dr. Anne Fausto-Sterling, Professor of Medical
Science, Brown University, for sharing these figures with us.

What sorts
of ongoing medical problems do intersexuals have?Is there a risk of gonadal tumors?

Dysgenetic testicular tissue (testicular tissue that has developed in an
unusual way) is at risk of developing tumors, and not merely because it
is undescended. That is, the risk persists even after successful orchiopexy
(surgically bringing undescended testes down into scrotal sac).

Ovarian tissue in intersexuals is not generally the cause of intersexuality,
is not dysgenetic, and does not appear to be at elevated risk of developing
tumors.

Undescended testes in women with AIS are at risk of developing tumors.

There are certain gonadal and adrenal tumors which produce hormones
and therefore intersexual expression. However, in this case the tumor causes
the intersexuality; the intersexuality does not cause the tumor.

In general, the likelihood of gonadal tumors is small (~5%) before mid-twenties,
and increases thereafter, with lifetime probabilities of 30% for partial
or complete gonadal dysgenesis, and 10% for 46XY true hermaphroditism.

Testosterone replacement in men with dysgenetic testes may increase
the probability of gonadal tumors developing.

To summarize,

Tumors are not likely in the absence of a Y chromosome (or
Y genes involved in testicular determination, which may be present on the
X chromosome in sex-reversal)

When there is a Y chromosome or Y genes are surmised to be present,
the gonads are at elevated risk, and should be carefully monitored. Monitoring
is easier to do if the gonads are brought down into the scrotum.

Because the risk is slight before early adulthood, gonadectomy should
not be imposed on infants. It should be delayed until the patient can weigh
the options and choose for her/himself. Functioning gonads, even partially
functioning gonads, are a big advantage over hormone replacement therapy.
The patient must be allowed to weigh the risks, talk with other patients
about their experiences, and choose what is best for her/himself. Note,
though, that it is critical to remove partially functioning testes before
puberty from an intersexual who identifies as female and wishes her body
not to virilize.

Much of this material (except the paragraph above!) comes from "Wilkins
The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence
4th edition," ed Kappy, Blizzard and Migeon, Baltimore: Charles C. Thomas,
1994.

Hormone replacement
therapy and osteoporosis

Sex hormones (principally testosterone or estrogen) are necessary to maintain
healthy adult bones. Persons born without functioning gonads, or whose
gonads have been removed, should be under an endocrinologist's care and
should maintain hormone replacement therapy for life.

Many intersexuals, having developed a distrust or aversion for medical
people, avoid medical care and drop hormone replacement therapy which was
prescribed during puberty. This can result in extreme osteoporosis (brittle
bones). Osteoporosis worsens silently, but at advanced stages it can destroy
your quality of life. Persons with advanced osteoporosis are vulnerable
to frequent bone fractures, especially of the spine, hip, and wrist. These
fractures can be caused by a small amount of force, and are extremely painful
and debilitating. Each spine fracture may put you flat on your back for
one to two months.

If you have been without gonads or hormone replacement therapy for years,
it is vital to get a bone density scan performed, to evaluate the condition
of your bones (a simple, non-invasive procedure using a specialized x-ray
machine), and to seek the advice of an endocrinologist in order to establish
a regimen of hormone replacement therapy that works for you. If you have
had bad experience in the past with hormones, we encourage you to find
an endocrinologist who will work with you to adjust the mix and schedule
of hormones until you find what works. If your bone density is low, your
endocrinologist will probably recommend calcium supplements and weight-bearing
exercise (not swimming!) to maintain density.

If your bone density scan is performed on a DEXA machine, make certain
to do any follow-up scans on the same machine, and with the same reader.

A number of drugs currently in the biomedical news may prove useful
for rebuilding lost bone density. If your bone density is low, check in
with a qualified specialist regularly for the latest information.

The danger of osteoporosis is considerably worse for intersexuals than
for post-menopausal women, because the intersexual will be without hormones
for many decades. Do not disregard this danger!

What are other support
groups?

ISNA provides this list of contacts for other support groups for intersexuality
as a service. The appearance of any group in this space does not constitute
an endorsement -- one or two have been reported to be reluctant to deal
with some important issues. Please do contact them with an open mind, and
learn both what they can offer you and what you can offer them!

Founded by a mother frustrated with the isolation and lack
of available information, HELP is a support group for parents, family,
friends, and persons affected by sex differentiation disorders, and a source
of medical information, literature, and personal experiences.

AGGPG (Arbeitsgruppe gegen Gewalt in der Paediatrie und Gynaecology
- translation: Workgroup Against Violence in Pediatrics and Gynecology)
is like ISNA, ISVO, GMSSN founded by intersexuals. Around some others our
specific subject is violence done to persons, whose sex is, according to
medical professions and society, not correct. We work against mutilation,
rape and other harmful "treatments" .We work for a realistic knowledge
about sex and gender variance as well as fighting for social acceptance.
AGGPG is a German group.

The AIS Support Group, based in England but with representatives
in the
United States and Canada, provides support to AIS women, their parents,
relatives and partners. It publishes a newsletter three times per year,
holds
semi-annual meetings (in England but with future meetings in the US
scheduled) and offers a variety of publications relevant to AIS.

Where can I read more?

Where can I read first
person writings of intersexuals?

The most important resource for reading intersexual writings is the publications
of ISNA, especially Hermaphrodites with Attitudes. Some ISNA members have
also written personal accounts of their intersexuality, available on the
Web at Intersex Voices.

Describes the life stories of Cheryl Chase and Morgan Holmes,
based on personal interviews. Photos of Holmes. Opinions of intersex specialists
Grumbach of UCSF and Gearhart of Hopkins (surgery is necessary to prevent
parents from treating child as an outcast) are contrasted with personal
experience of Chase and Holmes (surgery experienced as mutilation, causing
sexual dysfunction). Anne Fausto-Sterling criticizes intersex specialists
as unwilling to follow up patients to determine the outcome of their interventions.

"Once a dark secret," BMJ 1994; 308:542 (19 February).

A woman with XY karyotype and "testicular feminization" (androgen
insensitivity syndrome) briefly relates how damaging she has found the
secrecy surrounding her condition. "Mine was a dark secret kept from all
outside the medical profession (family included) but this is not an option
because it both increases the feelings of freakishness and reinforces the
sense of isolation. It also neglects the need for the counselling of siblings."

This letter responds to "Once a dark secret" in the 19 Feb
issue. The author discusses the issue of gender identity, criticizes the
secrecy and the labeling of women with complete androgen insensitivity
as "male" or "hermaphrodite."

"Be open and honest with sufferers," BMJ 1994 308:1042 (16 April).

The author of this letter has also been subjected to secrecy
surrounding her androgen insensitivity. This secrecy produced a "lifetime
of unnecessary secrecy, shame, delayed action, and great damage to my personal
and sexual identity and self esteem."

Holmes, Morgan. See entries under "WHERE CAN I READ DECONSTRUCTIONS
OF THE MEDICAL VIEWPOINT?"

Horowitz, Sarah. "Both and Neither," SF Weekly, February 1, 1995.

For generations, doctors have been "fixing" babies born with
ambiguous genitals. Now adult "intersexuals" wonder if their true identities
have been surgically mutilated. The article doesn't take sides, and plays
the "expert" doctors' opinions against our opinions. Needless to say, the
doctors insist that no one can be allowed to remain intersexual, and we
(Cheryl, Morgan, and David) assert that we are intersexual, and that we
have been harmed by medicalization. Anne Fausto-Sterling takes our side,
and Suzanne Kessler is "sympathetic" with ISNA's goals, but cautions that
what doctors are doing is enforcing a cultural mandate, and that doctors
are not likely to participate in a revolution.

Where can I read deconstructions
of the medical viewpoint?

Fausto-Sterling, Anne. "The Five Sexes: Why Male and Female are Not Enough,"
The Sciences, March/April 1993:20-24. Reprinted on New York Times Op-ed
page, March 12, 1993. See also the Letters from Readers in the July/August
1993 issue.

Fausto-Sterling questions the medical dogma that, without medical
intervention, hermaphrodites are doomed to a life of misery. What would
be the psychological consequences of raising children as unabashed intersexuals?
Imagine a society in which sexuality is celebrated for its subtleties and
not feared or ridiculed. The author's acceptance of the Victorian classification
of intersexuals as male, female, and true pseudo-hermaphrodites is unfortunate,
as is her naivite about the success of surgical intervention.

Ms Holmes, who was subjected during childhood to "clitoral
recession" surgery which removed most of her clitoris, analyzes the cultural
imperative to surgically alter intersexual children's genitals. "The medical
definition of what female bodies do not have and must not have: a penis.
Any body which does possess a penis must either be designated 'male' or
surgically altered. ... In the minds of doctors, bodies are for procreation
and heterosexual penetrative sex. ... I would have liked to have grown
up in the body I was born with, to perhaps run rampant with a little physical
gender terrorism instead of being restricted to this realm of paper and
theory. Someone else made the decision of what and who I would always be
before I even knew who and what I was."

Ms Kessler interviewed six medical specialists in pediatric
intersexuality to produce an account of the medical decision making process.
She describes the processes by which cultural assumptions about sexuality
in effect supersede objective criteria for gender assignment. Kessler concludes
that the key factor in making a decision is whether or not the infant has
a "viable" penis.

Ms Lee ananalyzes medical literature for clinical recommendations
concerning the diagnosis and treatment of intersexed infants, while invoking
deconstructive feminist theory to critique the medical "management" of
ambiguous genitalia. Her interdisciplinary approach places intersexuality
within a broader discourse of sex and gender, disputing the binary male/female
opposition as a social construction. Especially valuable is her transcription
of an interview with "Dr Y," an intersex specialist/clinician who acceded
to be interviewed about gender assignment only under the condition that
his identity be disguised.